Final Diagnosis -- Malakoplakia of allograft gastrointestinal tract including stomach, ileum, and colon and native colon


Malakoplakia of allograft gastrointestinal tract including stomach, ileum, and colon and native colon.


The patient's antibiotic treatment was continued and she improved clinically; she was discharged home six weeks after admission. Due to deconditioning associated with her prolonged hospital stay as well as new-onset motor axonal neuropathy which may be due to her immunosuppressive medication, she required outpatient physical therapy.


The features of the generalized intestinal histiocytic infiltrate present throughout our patient's entire gastrointestinal allograft are diagnostic of malakoplakia. The characteristic intracytoplasmic inclusions (Michaelis-Gutmann bodies) were found to be ubiquitous in the later biopsies and in retrospect, rare MG bodies were found in the previous set of biopsies.

Malakoplakia is a rare inflammatory condition which usually affects bladder mucosa (Voight, 1958) but can also involve the gastrointestinal tract (Terner and Lattes, 1965) and other sites. It is characterized by soft, pale yellow plaques on the mucosa. Malakoplakia is thought to result from the insufficient killing of gram negative bacteria by macrophages. The partially digested bacteria accumulate in macrophages along with iron and calcium deposits. These basophilic, often targetoid, inclusions are called Michaelis-Gutmann (MG) bodies (Michaelis and Gutmann, 1902). Malakoplakia is usually found in patients with a history of immunosuppression due to lymphoma, another malignant neoplasm, or transplant, most commonly renal transplant, and is treated with antibiotics.

Malakoplakia most commonly occurs in the urogenital tract, but the second most common site is the gastrointestinal tract. Less common sites include skin (Arul and Emmerson, 1977), breast (DiLeo and Anastasi, 1969), lungs and skeleton (Gupta, Schuster, and Christian, 1972) and brain (Takei, 1971). Gastrointestinal malakoplakia usually occurs in the colon and rectum, but has been reported in the stomach, duodenum, and appendix (Sekaran 2011, Kim 2003). It can be asymptomatic or present with diarrhea, abdominal pain, hemorrhage, or obstruction; involvement can be patchy or diffuse, with plaques, polyps, or a large mass. Gastrointestinal malakoplakia can be associated with gastrointestinal neoplasm (McClure, 1981), systemic disease such as chronic granulomatous disease, ulcerative colitis, tuberculosis, AIDS and celiac disease, or solid organ transplant (Yousif 2006, Keitel 2012, Mcclure 1981, Dasgupta, 1999). Malakoplakia usually occurs in adults, but it has previously been reported in children from 6 wks to 12 years old (McClure, 1981, Sekaran 2011, Kajbafzadeh, 2004), who often have associated systemic disease such as hereditary immunodeficiency. Occasionally malakoplakia can occur in immunocompetent children and adults with no significant medical history (Borghesi, 1997, Sekaran 2011).

Most of the solid organ transplants in malakoplakia cases are renal (Bae, 2013, Berney, 1999, Shah 2010, Yousif 2006, Keitel 2012). Post-transplant malakoplakia can occur either in the transplanted organ (Keitel 2012) or native organs. Gastrointestinal malakoplakia has previously been reported in children (Divya, 2010, Sekaran 2011, Krauel 2009, Kini 2005).

Gastrointestinal malakoplakia can be associated with solid organ transplant, especially when complicated by recurrent E coli infections (Yousif 2006, Keitel 2012, Mcclure 1981). Of note, our patient was admitted for E coli sepsis. Because malakoplakia is associated with immunosuppression, it is noteworthy that our patient had recently received additional immunosuppressive therapy for an episode of acute cellular rejection two months prior to admission. Our patient was receiving tacrolimus therapy, and gastrointestinal malakoplakia presenting with chronic diarrhea in an adult liver transplant patient receiving tacrolimus has been previously reported (Kim, 2007). As in our case, microscopy in previous cases has shown macrophages containing Gram-negative bacilli (McClure, 1981).

The etiology of malakoplakia is still not known; hypotheses include defective lysosomes and abnormal microtubular assembly similar to Chediak Higashi (Dasgupta, 1999). Phagocytes cannot kill bacteria without degranulation of lysosomes, for which normal microtubules are essential. Cholinergic agents and vitamin C, which help with phagocytic function, are a possible therapeutic adjunct in malakoplakia. Interestingly, our patient received her allograft for microvillus inclusion disease, which is a genetic myosin abnormality that leads to a congenital lack of apical microvilli in the epithelial cells of the small intestine as well as endosomal and lysosomal abnormalities. Although lysosomal abnormalities may have predisposed her to malakoplakia, the disease was the most severe in the allograft, not native, parts of her gastrointestinal system.

Malakoplakia can be silent (Bae, 2013) or present with severe clinical symptoms including intestinal perforation, sepsis, multiple organ failure, and death in adults (Berney, 1999). Malakoplakia can present with pseudotumor mass effects as well (Yousif 2006, Berney 1999). Malakoplakia can resolve completely when treated with extended course antibiotics (Yousif 2006). Our pediatric patient was very fortunate to survive severe malakoplakia with few sequalae. Though rare, malakoplakia should be on the differential in a transplant patient with abdominal symptoms and histocytic infiltrates on biopsy.


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Contributed by Martina Pejchal, MD, PhD and Jennifer Picarsic, MD

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